ABSTRACT: Although Chiari malformations are much more prevalent than once believed, no study has described treatment with an interventional spinal procedure. The purpose of this report was to discuss the clinical course of a patient who was diagnosed with a Chiari malformation and treated with three cervical epidural injections. In 2012, a 50-yr-old woman presented to a neurology clinic with chronic suboccipital headaches, diplopia, and increasing numbness/tingling in her upper extremities. Magnetic resonance imaging confirmed a type I Chiari malformation and a cervical syrinx. The patient was treated with three cervical epidural injections, after which her symptoms exacerbated. Consequently, a posterior fossa suboccipital craniectomy with C1 laminectomy and excision of extradural and intradural adhesions was performed. After surgical intervention, notable neurologic improvements were observed. Given the marked worsening of symptoms, the present report suggests that interventional spinal procedures may be a contraindication in the presence of a Chiari malformation with a syrinx.